Hemolytic uremic syndrome related to Shiga-like toxin-producing Escherichia coli with encephalitis hiding a human herpesvirus-6 infection: a case report

Abstract Background Cardiac and neurological involvement in hemolytic uremic syndrome are life-threatening complications. The most frequent complications of cardiac involvement in hemolytic uremic syndrome are myocarditis and cardiac dysfunction due to fluid overload. Pericarditis remains very rare in hemolytic uremic syndrome. To our knowledge, only five cases of cardiac tamponade associated with hemolytic uremic syndrome have been described in literature. Case summary A 27-month-old Caucasian girl presented with symptoms of nonbloody diarrhea and tonic-clonic seizures. The diagnosis of Shiga-like toxin-producing Escherichia coli hemolytic uremic syndrome with central nervous system involvement was made, and stool examination revealed infection with a Shiga-like toxin-producing Escherichia coli. She did not need renal replacement therapy but had severe neurological impairment. The patient’s course was complicated by pericardial effusion. A pericardiocentesis was performed via an apical approach because the pericardial effusion was predominantly surrounding the left ventricle. Effusion analysis showed an exudate and positivity for human herpesvirus-6B on polymerase chain reaction with viremia. This finding was consistent with primary human herpesvirus-6 infection with encephalitis. Conclusion We report this uncommon case of Shiga-like toxin-producing Escherichia coli hemolytic uremic syndrome associated with a severe human herpesvirus-6 infection. Secondary isolated pericardial effusion and atypical neurological involvement are uncommon in Shiga-like toxin-producing Escherichia coli hemolytic uremic syndrome and should lead the physician to perform additional investigations.

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Eculizumab Use in a Temporarily Dialysis-Dependent Patient With Shiga Toxin–Producing Escherichia Coli Hemolytic Uremic Syndrome With Neurological Complications

The Journal of Pediatric Pharmacology and Therapeutics ◽

10.5863/1551-6776-27.1.90 ◽

2021 ◽

Vol 27 (1) ◽

pp. 90-95

Author(s):

Bo Weber ◽

Dominic Chan ◽

Sandy Hammer

Keyword(s):

Escherichia Coli ◽

Renal Failure ◽

Hemolytic Uremic Syndrome ◽

Shiga Toxin ◽

Dose Adjustment ◽

Inpatient Rehabilitation ◽

Cellular Damage ◽

Neurological Involvement ◽

Organ Systems ◽

Uremic Syndrome

Shiga toxin–producing Escherichia coli hemolytic uremic syndrome (STEC-HUS) is the most common cause of acute renal failure in children, and it is associated with thrombocytopenia and hemolytic anemia. Although this disease primarily affects the kidney, it can also contribute to cellular damage in other organ systems, such as the CNS. Eculizumab is a monoclonal antibody that binds to complement proteins to prevent complement-mediated intravascular hemolysis in atypical HUS. In STEC-HUS, complement activation also occurs by Shiga toxin, and previous cases of eculizumab use in the setting of neurological involvement have been shown to be successful. We report the successful use of eculizumab in the setting of typical STEC-HUS–induced neurological symptoms including seizure, altered mental status, and left arm weakness. The patient also experienced concomitant renal failure requiring dose adjustment for hemodialysis. Following 2 doses of eculizumab, our patient was discharged to an inpatient rehabilitation facility with resolution of her renal injury, seizures, and altered mentation without adverse effects from eculizumab throughout the admission. Based on our case study, it appears that eculizumab may be given during or between hemodialysis without dose adjustment.

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A pediatric neurologic assessment score may drive the eculizumab-based treatment of Escherichia coli-related hemolytic uremic syndrome with neurological involvement

Pediatric Nephrology ◽

10.1007/s00467-018-4112-2 ◽

2018 ◽

Vol 34 (3) ◽

pp. 517-527 ◽

Cited By ~ 7

Author(s):

Paolo Giordano ◽

Giuseppe Stefano Netti ◽

Luisa Santangelo ◽

Giuseppe Castellano ◽

Vincenza Carbone ◽

...

Keyword(s):

Escherichia Coli ◽

Hemolytic Uremic Syndrome ◽

Neurological Involvement ◽

Assessment Score ◽

Uremic Syndrome

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Human herpesvirus 6: report of emerging pathogen in five patients with HIV/AIDS and review of the literature

Revista da Sociedade Brasileira de Medicina Tropical ◽

10.1590/s0037-86822011000400026 ◽

2011 ◽

Vol 44 (4) ◽

pp. 522-525 ◽

Cited By ~ 11

Author(s):

Marcelo Corti ◽

Maria Florencia Villafañe ◽

Norberto Trione ◽

Lilia Mamanna ◽

Belén Bouzas

Keyword(s):

Central Nervous System Involvement ◽

Human Herpesvirus ◽

Review Of The Literature ◽

Human Herpesvirus 6 ◽

Emerging Pathogens ◽

Emerging Pathogen ◽

Chain Reaction ◽

Polymerase Chain ◽

Herpesvirus 6 ◽

Hiv Aids

The reactivation of human herpesvirus 6 (HHV-6) in patients with AIDS can result in an acute and severe diffuse meningoencephalitis. We describe the epidemiological, clinical and outcome findings of five patients with diagnosis of HIV/AIDS and central nervous system involvement (CNS) due to HHV-6. Fever was present in all the patients. Meningeal compromise, seizures and encephalitis were present in some of the patients. Polymerase chain reaction (PCR) of cerebrospinal fluid (CSF) specimens was positive for HHV-6 in all the patients. HHV-6 should be included among opportunistic and emerging pathogens that involve the CNS in patients with AIDS.

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Peripheral neurological involvement in Shiga toxin-producing Escherichia coli-hemolytic uremic syndrome (STEC-HUS) as a rare complication with a positive outcome in two consecutive children – Case Report

10.21203/rs.3.rs-242540/v1 ◽

2021 ◽

Author(s):

Luisa Santangelo ◽

Giuseppe Stefano Netti ◽

Diletta Domenica Torres ◽

Giovanni Piscopo ◽

Vincenza Carbone ◽

...

Keyword(s):

Escherichia Coli ◽

Hemolytic Uremic Syndrome ◽

Functional Recovery ◽

Shiga Toxin ◽

Rehabilitation Program ◽

Lower Limbs ◽

Motor Deficit ◽

Neurological Involvement ◽

Renal Complication ◽

Uremic Syndrome

Abstract Background. The Neurological involvement is the most common extra-renal complication of Shiga toxin-producing Escherichia coli-hemolytic uremic syndrome (STEC-HUS). On brain magnetic resonance examination, main neurological signs encompass acute lesions of the basal ganglia and the white matter, which could usually regress after Eculizumab infusion. In contrast, STEC-HUS-related peripheral nervous system (PNS) involvement is very rare and, when occurring, it requires a careful management of neurological sequelae and an intensive multidisciplinary neuro-rehabilitation program.Case presentation. Here, we present two cases of severe and complicated STEC-HUS patients with PNS-involvement who successfully required therapeutic treatment and an intensive multidisciplinary neuro-rehabilitation program.In both cases, PNS-involvement followed the recovery from STEC-HUS-related severe central neurological damage and manifested mainly with marked bilateral motor deficit and hyporeflexia/areflexia in the lower limbs. The peripheral polyneuropathy was treated with immune-suppressive therapy (methylprednisolone pulses, i.v. immunoglobulins, therapeutic plasma exchange), followed by a prolonged intensive neuro-rehabilitation program. After 8 months of neuro-rehabilitation, both patients gained complete functional recovery.Conclusions. PNS involvement during STEC-HUS is a rare event and potentially leading to severe disability. A timely clinical assessment is mandatory to set up a prompt therapeutic and neuro-rehabilitation program and to obtain a complete clinical and functional recovery.

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